Page 36 - MEMOCiberes014-ENG

and extracellular-dependent microenvironment . Although EnMT has been less studied, it has been included as one of the different cellular process characteristics in the remodelling of pulmonary hypertension (PH)-associated IPF, together with endothelial dysfunction . PH in IPF portends a poor prognosis .
ADVANCED 3D CELL CULTURE MODEL FOR THE STUDY OF CELL-ECM PROTEIN INTERACTIONS
Traditional approaches to study IPF include conventional 2D culture systems, which lack essential components of the original tissue, and animal models that contain the full complexity of the tissue but lack the irreversible behaviour of IPF . There is wide evidence that cells grown in 3D cultures with appropriate ECM components retain many of their phenotypic characteristics, thereby behaving more closely to their in vivo conditions . Our programme is working in a 3D culture model.
Aims and objectives
1 . To study glycoprotein effect on primary human lung alveolar and mesenchymal cells (migration, metabolism and differentiation) and the regulation of its synthesis in fibro- myofibroblasts .
2 . To evaluate the differences in cell behaviour and experimental results depending on telomere length and telomerase gene mutations .
3 . To identify, characterize and assess, in vitro, the functional status and the regenerative/ reparative capacity of both hLRSC, including lung-hMSCs and BM-hMSCs, from patients with IPF, compared to those obtained from subjects with normal lung function .
4 . Compare in these cells released factors related to signalling pathways that induce proliferative and fibrogenic features of pulmonary target cells .
5 . To investigate the remodelling of those vascular structural cells from patients with PH complicating IPF, and its possible regulation through some inhibitors .
6 . To study aquoporin system in lung fibrosis and its modulation depending on the cell type and environmental growing conditions .
Main results until the end of 2014
In reference to CIBERES CRPs, the Pulmonary Fibrosis Programme highlights the following as its relevant results for the scientific and clinical community:
Interstitial pulmonary diseases constitute a group of rare or minority diseases affecting the interstitium and overall account for 15% of all respiratory diseases, being the most prevalent and lethal being idiopathic pulmonary fibrosis (IPF) .
The incidence and prevalence of IPF has increased in recent years (3-6 .8 /100000/year and 13-20/100000/ year, respectively), and other fibrosing pulmonary diseases have been detected which, although more slowly, also lead to respiratory failure and death of the patient . Mean survival media in IPF is 3-5 years from diagnosis and there is still no cure or treatment that allows stopping the progression of the disease . However, recent advancements in the knowledge on the pathogenesis of the disease and clinical trials conducted up until now, in which the coordinated Pulmonary Fibrosis programme has contributed, has led to the appearance of new anti-fibrotic treatments, nintedanib and Pirfenidone, which slow the progression of the disease and have completely modified the therapeutic approach of these patients (published clinical trial results, new drugs and therapeutic guidelines) .
The objective of the current Pulmonary Fibrosis research SP is to understand some still unknown and relevant pathogenic aspects of pulmonary fibrogenesis associated with accelerated ageing as a predisposing factor in order to try to stop the progression or even prevent the development of the disease . Initial results have
36 CIBERES » Annual report 2014